Melkersson-Rosenthal Syndrome

What is Melkersson-Rosenthal Syndrome?

Melkersson Rosenthal syndrome, also known as Melkersson syndrome, MROS and MRS, is a rare disorder that develops during childhood and early adolescence. The most common signs are recurrent bouts of facial paralysis (also known as palsy), inflammation in the face and lips and the appearance of folds and fissures on the tongue. Some people suffer from all these symptoms, while others may only display one or two signs.

Related conditions

Syndromes and conditions that are related to Melkersson Rosenthal syndrome include:

• Bell’s palsy: this is a progressive neurological condition that affects the 7th cranial nerve. Symptoms include facial paralysis on one side of the face, ear pain and fever that arises from compression of the nerve or disturbed blood flow to the 7th cranial nerve. The cause is not known and experts believe there may be multiple causes, including viral infections, genetic links and conditions that affect immunity.
• Crohn’s disease: Crohn’s disease is an inflammatory condition that affects the bowel and causes symptoms such as abdominal cramps, diarrhoea, weight loss and a high temperature.
• Sarcoidosis: sarcoidosis is a condition that affects several areas of the body. It is characterised by the development of several granulomas and collections of white blood cells that can cause problems with the function of the organs. The lungs are most commonly affected, but the lymph nodes, eyes, liver, heart, spleen and skin may also be implicated.

What causes Melkersson Rosenthal syndrome?

It is thought that Melkersson Rosenthal syndrome is linked to genetic causes. However, no specific gene has been identified as yet. In some cases, MRS many be linked to other health conditions, including Crohn’s disease.

Who is affected?

Melkersson Rosenthal syndrome is slightly more common in females than males and it tends to develop during childhood and the adolescent or early adult years. The exact number of cases is unknown.

What are the symptoms?

The first symptom that becomes apparent is often inflammation of the lips, cheeks and eyelids. Sometimes, one side of the scalp may also feel swollen. Initially, periods of inflammation are short-lived, but they tend to get progressively longer. In some cases, swelling may become permanent.

The lips may also appear sore and cracked and additional symptoms may include disturbed vision, a high temperature and headaches.

Around 20-40 percent of people with MRS have a furrowed tongue and facial paralysis affects around 30 percent of individuals. Often, facial paralysis develops shortly after the lips become inflamed. It can affect one or both sides and may be temporary, or less commonly, permanent.

How is Melkersson Rosenthal syndrome diagnosed?

Medical history and symptoms are usually sufficient to make a diagnosis. However, a biopsy from the lips may be ordered to confirm the diagnosis and rule out other potential problems, such as Crohn’s disease.

Treating Melkersson Rosenthal syndrome

In most cases, symptoms subside without the need for treatment, however without treatment, episodes of swelling and paralysis may become longer and more severe. Treatment options include steroid medication, anti-inflammatory drugs (non-steroid) and antibiotics. If the lips are extremely swollen, surgery or radiation therapy may be recommended.

Exercises to strengthen the facial muscles and reduce inflammation may also be helpful. This kind of facial rehabilitation is similar to treatment used for Bell’s palsy.